What is Dementia with Lewy Bodies (DLB)?

From the Guidelines

Lewy Body dementia is a progressive brain disorder that requires early diagnosis and proper management to improve quality of life, and treatment typically involves medications like cholinesterase inhibitors at lower doses than for Alzheimer's, as well as non-pharmacological interventions like physical, occupational, and speech therapy. Lewy Body dementia is the second most common type of dementia after Alzheimer's disease, affecting approximately 1.4 million Americans [ 1 ]. Symptoms include cognitive fluctuations (varying alertness and confusion), visual hallucinations, movement problems similar to Parkinson's disease (tremors, stiffness, slow movement), sleep disturbances (acting out dreams during REM sleep), and autonomic dysfunction affecting blood pressure and digestion.

Key Considerations in Management

• Treatment should prioritize improving cognition and managing symptoms, with a focus on quality of life [ 1 ].
• Cholinesterase inhibitors like rivastigmine and donepezil are commonly used, but at lower doses than for Alzheimer's due to sensitivity [ 1 ].
• Antipsychotics should generally be avoided due to the risk of severe reactions [ 1 ].
• Non-pharmacological interventions such as physical, occupational, and speech therapy are crucial in managing physical symptoms and improving quality of life [ 1 ].

Disease Progression and Caregiver Support

• The disease typically progresses over 5-8 years, though this varies widely [ 1 ].
• Early diagnosis is crucial for proper management, as treatments differ from other dementias [ 1 ].
• Caregivers should establish consistent routines, minimize stress, and ensure a safe environment as the disease progresses [ 1 ].
• Deprescription of anti-dementia drugs should be considered based on individual patient factors, including worsening of dementia, lack of benefit, severe side effects, and poor medication adherence [ 1 ].

From the Research

Overview of Lewy Body Dementia

• Dementia with Lewy bodies (DLB) is a complex disease that involves a variety of cognitive, behavioral, and neurological symptoms, including progressive memory loss, visual hallucinations, parkinsonism, cognitive fluctuations, and rapid eye movement sleep behavior disorder (RBD) 2.
• DLB is the third most common of all the neurodegenerative diseases, behind Alzheimer's disease and Parkinson's disease (PD), with a median age of onset of 76.3 years 2.
• The clinical presentation and course of DLB can vary widely, with most patients having variable amounts of Alzheimer's changes in their brains 2.

Symptoms and Diagnosis

• Characteristic symptoms of DLB include dementia, marked fluctuation of cognitive ability, early and persistent visual hallucinations, and spontaneous motor features of parkinsonism 3.
• Falls, syncope, transient disturbances of consciousness, neuroleptic sensitivity, and hallucinations in other modalities are also common 3.
• Diagnosis of DLB can be challenging, particularly at early stages of the disease, and in patients with mixed neuropathological changes 4.
• New diagnostic criteria and specific diagnostic biomarkers can help improve detection rates and diagnostic accuracy 4.

Treatment and Management

• Cholinesterase inhibitors, such as rivastigmine, galantamine, and donepezil, have been shown to be effective in managing the cognitive and behavioral symptoms of DLB 2, 3.
• Memantine can improve clinical global impression of change in patients with mild to moderate DLB 2.
• Levodopa can treat the parkinsonism of some DLB patients, but the dose is often limited due to the risk of agitation or worsening of visual hallucinations 2.
• Other treatments, such as zonisamide, valproic acid, and pimavanserin, may also be effective in managing specific symptoms of DLB 2.

Patient and Caregiver Experience

• People living with Lewy body dementia and their family carers often experience difficulty finding information and support around diagnosis, disease progression, and managing complex symptoms 5.
• Delayed diagnosis and complex symptom burden can lead to increased caregiver burden, grief, and stress, and reduced quality of life 5.
• Current evidence is largely quantitative, with a focus on family caregiver burden and unmet need, highlighting the need for more holistic research on the lived experience of people with Lewy body dementia and their families 5.